Open Versus Ultrasound Guided Tunneled Central Venous Access in children: A Randomized Controlled Study.

BACKGROUND: The purpose of this study was to compare open insertion to ultrasound guided percutaneous insertion of central access catheters performed in a tertiary pediatric hospital in terms of its safety and complication rates. METHODS: This was an ethics approved prospective randomized trial of children under 16 y of age. Procedure was performed by surgeons with varying experience with percutaneous and open insertion. Primary outcome studied was complications-immediate and late. Secondary outcomes were time taken to complete procedure, conversion rates, duration of line use. RESULTS: A total of 108 patients were analyzed. Sixty-four were male. Right internal jugular vein was accessed in 97. Eighty-one lines were double lumen, 23 implantable access devices, and the rest were single lumen catheters. More than one needle puncture was needed in 22% of the cases but there were no conversions in the ultrasound group. Twelve patients needed more than one insertion to achieve optimal position of the tip. Eleven patients had immediate and late complications. Percutaneous lines lasted 45 d longer though this was not statistically significant. Operating time was 20.6% shorter with percutaneous access. Post-removal measurement of vein size by ultrasound demonstrated significant decrease in size in the open group. CONCLUSIONS: Ultrasound guided percutaneous insertion was safe. The study also demonstrated a decrease in operating times, preservation of vein size, and no increase in complication rates in the US group when performed by operators of varying expertise.

The Predictors for Positive Yield Abdominal Computed Tomography in Pediatric Abdominal Trauma.

OBJECTIVES: Abdominal computed tomography (ACT) use in the initial evaluation of pediatric abdominal trauma is liberal in most instances. The aim of this study was to identify the predictors for a positive yield ACT scan in this population. METHODS: A prospective, cohort, single-center observational study was conducted at Children's Hospital at Westmead, New South Wales, from January 2008 to June 2015 on 240 pediatric abdominal trauma patients who had abdominal computed tomography. Clinical, laboratory, imaging, and interventional variables were explored with univariate and multivariate analyses among children who sustained abdominal trauma. RESULTS: Of 240 patients, positive ACT scans were found in 161 patients (67%), 112 patients (47%) had intra-abdominal injury, and 20 patients (8%) required invasive therapeutic interventions. Mortality rate was 1.7% (4 patients) due to nonabdominal causes. Multivariate analyses revealed that increasing age (odds ratio [OR], 1.12; 95% confidence interval [CI], 1.02-1.24; P = 0.024), high injury severity score (OR, 1.14; 95% CI, 1.07-1.21; P < 0.001), abnormal abdominal examination (OR, 5.95; 95% CI, 2.08-17.01; P = 0.001), elevated alanine aminotransferase greater than 125 IU/L (OR, 46.28; 95% CI, 2.81-762.49; P = 0.007), abnormal pelvic radiograph (OR, 14.03; 95% CI, 2.39-82.28; P = 0.003), presence of gross hematuria (OR, 4.14; 95% CI, 1.04-18.23; P = 0.044), low initial hematocrit level (less than 30%) (OR, 8.51; 95% CI, 1.14-63.70; P = 0.037), and positive focused assessment with sonography for trauma (OR, 2.61; 95% CI, 1.01-7.28; P = 0.048) remained significantly associated with abnormal ACT scan. In contrast, those who required scanning of other body region(s) were less likely to have abnormal ACT scan (OR, 0.34; 95% CI, 0.14-0.86; P =0.022). CONCLUSIONS: Integrating the abdominal examination findings, relevant laboratory values, and focused assessment with sonography for trauma results with the physicians' suspicion may aid in stratifying patients for ACT scan. Further efforts should be made to decrease number of normal ACT scans; yet not to increase the number of delayed or missed injures with its inherent morbidity and mortality.

Neurocognitive outcomes in children following immersion: a long-term study.

OBJECTIVE: To investigate long-term neurocognitive outcomes after a near-drowning incident in children who were deemed neurologically intact on discharge from hospital. DESIGN: A prospective cohort study of near-drowning children. SETTING: 95 drowning and near-drowning admissions, 0-16 years of age, from January 2009 to December 2013, to The Children's Hospital at Westmead, Sydney, NSW, Australia. PARTICIPANTS: 23 children both met the criteria and had parental consent for the study. MAIN OUTCOME MEASURES: Identification of the long-term deficits in behaviour, executive function, motor skills, communicative skills and well-being over a 5-year period. Assessment was undertaken at 3-6 months, 1 year, 3 years and 5 years after near-drowning at clinic visits. Physical developmental screening and executive function screening were done using Behavior Rating Inventory of Executive Function-Preschool version (BRIEF-P) and BRIEF. RESULT: 95 drowning and near-drowning episodes occurred during the study period. 10 (11%) children died, 28 were admitted to the paediatric intensive care unit and 64 directly to a ward. 3 children died in emergency department, 7 children had severe neurological deficit on discharge from the hospital. 23 were subsequently recruited into the study; 5 (22%) of these children had abnormalities in behaviour and/or executive function at some during their follow-up. CONCLUSION: Children admitted to hospital following a near-drowning event warrant long-term follow-up to identify any subtle sequelae which might be amenable to intervention to ensure optimal patient outcome.

Transition zone pull-through in Hirschsprung's disease: a tertiary hospital experience.

BACKGROUND: Pull-through of ganglionic bowel is essential for successful treatment of Hirschsprung's disease. We studied the incidence of transition zone pull-through in our institution and compared its outcome with ganglionic bowel pull-through. METHODS: Children who underwent Soave's pull-through for Hirschsprung's disease from January 2005 to November 2012 were studied. Patients were divided into two groups: ganglionic bowel pull-throughs (Group 1) and transition zone pull-throughs (Group 2). Demographics, presentations, surgical procedure, post-operative results and complications including redo procedures were recorded and reviewed along with histopathology reports. RESULTS: Fifty patients underwent Soave's pull-through for Hirschsprung's disease in our group. The median age at surgery was 13.5 days in Group 1 and 22.5 days in Group 2. Transition zone pull-through occurred in eight children (16%). Transition zone pull-through was attributed to errors in histologic interpretation (n = 5), sampling (n = 2) and surgical technique (n = 1). The transition zone was significantly longer in Group 2 (P = 0.002). Constipation and enterocolitis were the main complications needing therapy. One child in Group 2 required surgery for adhesive intestinal obstruction. CONCLUSIONS: The length of the transition zone in children with transition zone pull-through was significantly longer. Though our children with transition zone pull-through did not require redo surgery the possibility of redo surgery remains. Transition zone pull-through should still be considered an error and should be prevented.

Aganglionosis with the absence of hypertrophied nerve fibres predicts disease proximal to rectosigmoid colon.

PURPOSE: The gold standard for the diagnosis of Hirschsprung's disease (HSCR) is the pathologic evaluation of a rectal biopsy that demonstrates the absence of ganglion cells and nerve fibre hypertrophy. However, it has been frequently reported that hypertrophic nerves may not be present in some variants like long-segment HSCR, total colonic aganglionosis, premature and very young infants. The aim of this study was to determine this association. METHODS: We performed a retrospective review of the HSCR database at our tertiary care children's hospital from 2000 to 2013. In order to analyse the relationship between the diameter of the nerve fibres and the level of aganglionosis, we classified the patient sample into two groups-fibres ≤40 and >40 µm. The groups were statistically compared with P < 0.05 being significant. RESULTS: Rectal biopsies of 92 patients confirmed as HSCR with definitive operation performed at the same institution were reviewed. The mean nerve diameter was 50.1 µm (range 20-87.5 µm). Nerve fibre diameter ≤40 µm was predictive of transition zone above the sigmoid colon. A specificity of 77.3 % and a likelihood ratio of 2.03 supported this perception. No correlation was noted between nerve fibre diameter and gestational age at birth, birth weight or age at biopsy. CONCLUSION: The absence of nerve fibre hypertrophy in the presence of aganglionosis on rectal biopsy specimens is predictive of long-segment HSCR.

Actinomycin D Specifically Reduces Expanded CUG Repeat RNA in Myotonic Dystrophy Models.

Myotonic dystrophy type 1 (DM1) is an inherited disease characterized by the inability to relax contracted muscles. Affected individuals carry large CTG expansions that are toxic when transcribed. One possible treatment approach is to reduce or eliminate transcription of CTG repeats. Actinomycin D (ActD) is a potent transcription inhibitor and FDA-approved chemotherapeutic that binds GC-rich DNA with high affinity. Here, we report that ActD decreased CUG transcript levels in a dose-dependent manner in DM1 cell and mouse models at significantly lower concentrations (nanomolar) compared to its use as a general transcription inhibitor or chemotherapeutic. ActD also significantly reversed DM1-associated splicing defects in a DM1 mouse model, and did so within the currently approved human treatment range. RNA-seq analyses showed that low concentrations of ActD did not globally inhibit transcription in a DM1 mouse model. These results indicate that transcription inhibition of CTG expansions is a promising treatment approach for DM1.

Comparative analysis of trends in paediatric trauma outcomes in New South Wales, Australia.

Paediatric trauma centres seek to optimise the care of injured children. Trends in state-wide paediatric care and outcomes have not been examined in detail in Australia. This study examines temporal trends in paediatric trauma outcomes and factors influencing survival and length of stay. A retrospective review was conducted using data from the NSW Trauma Registry during 2003-2008 for children aged 15 years and younger who were severely injured (injury severity score >15). To examine trauma outcomes descriptive statistics and multivariable logistic and linear regression were conducted. There were 1138 children severely injured. Two-thirds were male. Road trauma and falls were the most common injury mechanisms and over one-third of incidents occurred in the home. Forty-eight percent of violence-related injuries were experienced by infants aged less than 1 year. For the majority of children definitive care was provided at a paediatric trauma centre, but less than one-third of children were taken directly to a paediatric trauma centre post-injury. Children who received definitive treatment at a paediatric trauma centre had between 3 and 6 times higher odds of having a survival advantage than if treated at an adult trauma centre. The number of severe injury presentations to the 14 major trauma centres in NSW remains constant. It is possible that injury prevention measures are having a limited effect on severe injury in NSW. This research provides stimulus for change in the provision and co-ordination in the delivery of trauma care for injured children.

Infantile hypertrophic pyloric stenosis: has anything changed?

AIM: The study aims to review the contemporary presentation, diagnosis and treatment of children with infantile hypertrophic pyloric stenosis (IHPS) at an Australian paediatric tertiary centre. METHODS: A retrospective case review of patients with IHPS presenting to our institution between January 2004 and December 2010 was performed. Results were compared with a previous study evaluating two earlier 6-year series of patients treated between January 1984 and November 1995. RESULTS: Of 362 infants presenting over the current 7-year review, 84.8% were male. Mean age of admission fell from 5.9 weeks in the previous study to 5.4 weeks. An 'olive' was palpated on examination in 48%, visible peristalsis seen in 25% and 16% of patients presented with haematemesis. Diagnosis solely on clinical examination fell from 74% to 9% and was associated with a corresponding increase in use of ultrasound from 16% to 91%. The frequency of serum chloride values less than 85 mmol/L declined from 26% to 9%. A variety of open and minimally invasive surgical approaches were used with similar outcomes, although laparoscopic pyloromyotomy was associated with significantly higher rates of wound infection (χ(2) = 4.6, P = 0.03). The frequency of major complications remained low at 1%. CONCLUSION: Contemporary patients with IHPS typically present earlier with a reduction in the incidence of metabolic derangement. Diagnosis based on clinical examination alone appears uncommon, with the majority of suspected cases confirmed by ultrasound. There was no clear difference in overall outcome based on the surgical approach used, although minor variations may reflect our institution's initial experience with laparoscopic pyloromyotomy.

Off-road vehicle trauma in children: a New South Wales perspective.

OBJECTIVE: There is paucity of data on off-road vehicle injuries in children in Australia. We performed a retrospective study from 1998 to 2003 to analyze the frequency and nature of injuries in children involved in off-road vehicle crashes in the state of New South Wales. METHODS: Medical records were identified from search of the trauma database and hospital medical records database for off-road (all-terrain) vehicles. RESULTS: A total of 271 children were identified, 86% of whom were boys. The mean age was 10 years (range, 2-16 years); and the mean length of stay, 5.8 (9) days (range, 1-40 days). The mean injury severity score was 6 (5.9). Most were drivers (85%). Injury mechanism was falls in 161; collision with stationary object, 54; moving object, 4; rollovers, 7; and others, 8. Eighty-four percent were on 2 wheelers, whereas 11% were quad bikes, and the rest were on tricycles or other vehicles. Distribution of the body region injured was head and neck in 66 patients; face, 51; chest, 25; abdomen, 36; pelvis, 5; spines, 14; upper limbs, 96; and lower limbs, 116. Only 55% were helmeted at the time of the incident. Sixty-five percent of these children required surgical treatment. Most were fractures (98) followed by soft tissue injuries (49). Seventeen had posthead injury sequelae requiring rehabilitation support, and 21 required multiple surgeries. There were 7 deaths during the study period in New South Wales. CONCLUSIONS: Off-road motor vehicle injuries are a significant problem in children. There are no legal safety regulations for use of these vehicles. With the increasing sales of these vehicles, the incidence of injury may rise. There seems a need for education and legislation in relation to the safety issues concerned with these vehicles.

Catheter balloon-related urethral trauma in children.

AIM: To review urethral injuries arising from incorrect balloon inflation in children undergoing urinary catheterisation. METHOD: Retrospective review from 1995-2006. Children who sustained catheter-related injury at The Children's Hospital at Westmead were identified through medical records database and reviewed. RESULTS: Six patients were identified over the 11-year period. All six were boys. Age ranged from <1 month to 16 years. All but one occurred in hospital. All injuries were confirmed by urethrogram. Bulbar and prostatic urethra was involved in an equal number of children studied. Three patients required suprapubic catheters. Follow-up imaging revealed healing without stricture in all patients. CONCLUSION: Balloon-related urethral trauma can be avoided by educating health-care professionals on proper placement and confirmation of position of catheter. Though there were no long-term complications noted, a temporary suprapubic diversion may be needed.

Successful treatment of recurrent epididymo-orchitis: Laparoscopic excision of the prostatic utricle.

Prostatic utricle presenting with recurrent epididymo-orchitis is not uncommon. Excision of prostatic utricle is the treatment of choice. The various techniques described in literature suffer from the disadvantages of incomplete excision due to poor view. We report the successful laparoscopic excision of prostatic utricle in childhood.

Deficiency in endothelin receptor B reduces proliferation of neuronal progenitors and increases apoptosis in postnatal rat cerebellum.

Endothelins regulate cellular functions in the mammalian brain through the endothelin receptors A and B (EDNRA and EDNRB). In this study, we investigated the role of EDNRB on cell proliferation in the cerebellum by using the spotting lethal (sl) rat, which carries a naturally occurring deletion in the EDNRB gene. Proliferating cells in the three genotypes, wild-type (+/+), heterozygous (+/sl) and homozygous mutant (sl/sl) rats were labelled by intraperitoneal injection of 5-bromo-2'-deoxyuridine (BrdU) at postnatal day 2. The density of BrdU-positive cells (per mm(2)) in the external germinal layer of sl/sl rats (Mean +/- SEM, 977 +/- 388) was significantly reduced compared to +/+ (4915 +/- 631) and +/sl (2304 +/- 557) rats. Subsequently, we examined the effects of EDNRB mutation on neural apoptosis by terminal deoxynucleotidyltransferase-mediated dUTP nick end-labelling assay. This showed that the density of apoptotic cells in the cerebella of sl/sl rats (9.3 +/- 0.5/mm(2)) was significantly more increased than +/+ rats (4 +/- 0.7). The expression of brain-derived neurotrophic factor (BDNF) and glial cell line-derived neurotrophic factor (GDNF) were measured with standard ELISA, but were unchanged in all genotypes. These results suggest that ENDRB mediates neural proliferation and have anti-apoptotic effects in the cerebellum of the postnatal rat, and that these effects are independent of changes in the expression of BDNF and GDNF. Our findings will lead to better understanding of the morphological changes in the cerebellum of Hirschsprung's disease patients with congenital EDNRB mutation.

Unusual neck sinus--first or second cleft?

A sinus tract presenting with an opening around the angle of mandible is suggestive of first cleft remnant. We present the case of a 4-year-old boy with a recurrent discharging sinus around the angle of the right mandible whose internal opening was near the tonsil on imaging. Complete excision was performed with facial nerve monitoring. We discuss technical aspects of the surgery and possible embryology.

Posttraumatic small bowel obstruction in children.

BACKGROUND: The diagnosis of intestinal injuries in children after blunt abdominal trauma can be difficult and delayed. Most children who suffer blunt abdominal trauma are managed nonoperatively, making the diagnosis of intestinal injuries more difficult. We sought to gain information about children who develop intestinal obstruction after blunt abdominal trauma by reviewing our experience. METHODS: Review of records from a pediatric tertiary care center over an 11.5-year period revealed 5 patients who developed small bowel obstruction after blunt trauma to the abdomen. The details of these patients were studied. RESULTS: All patients were previously managed nonoperatively for blunt abdominal trauma. Intestinal obstruction developed 2 weeks to 1 year (median, 21 days) after the trauma. Abdominal x-ray, computerized tomography scan, or barium meal studies were used to establish the diagnosis. The pathology was either a stricture, an old perforation, or adhesions causing the intestinal obstruction. Laparotomy with resection and anastomosis was curative. CONCLUSIONS: Posttraumatic small bowel obstruction is a clinical entity that needs to be watched for in all patients managed nonoperatively for blunt abdominal trauma.

Evaluation of the thyroid transcription factor-1 gene (TITF1) as a Hirschsprung's disease locus.

Hirschsprung's disease (HSCR, colonic aganglionosis) is an oligogenic entity that usually requires mutations in RET and other interacting loci. Decreased levels of RET expression may lead to the manifestation of HSCR. We previously showed that RET transcription was decreased due to alteration of the TITF1 binding site by two HSCR-associated RET promoter single nucleotide polymorphisms (SNPs). This prompted us to investigate whether DNA alterations in TITF1 could play a role in HSCR by affecting the RET-regulatory properties of the TITF1 protein. Our initial study on 86 Chinese HSCR patients revealed a Gly322Ser amino acid substitution in the TITF1protein. In this study we have examined an additional 102 Chinese and 70 Caucasian patients, and 194 Chinese and 60 Caucasian unselected, unrelated, subjects as controls. The relevance of the DNA changes detected in TITF1 by direct sequencing were evaluated using bioinformatics, reporter and binding-assays, mouse neurosphere culture, immunohistochemistry and immunofluorescence techniques. Met3Leu and Pro48Pro were identified in 2 Caucasian patients and 1 Chinese patient, respectively. In vitro analysis showed that Met3Leu reduced the activity of the RET promoter by 100% in the presence of the wild-type or HSCR-associated RET promoter SNP alleles. The apparent binding affinity of the TITF1 mutated protein was not decreased. The Met3Leu mutation may affect the interaction of TITF1 with its protein partners. The absence of Titf1 expression in mouse gut but not in human gut suggests that the role of TITF1 in gut development differs between the two species. TITF1 mutations could contribute to HSCR by affecting RET expression through defective interactions with other transcription factors.

Transfer of pediatric trauma patients to a tertiary pediatric trauma centre: appropriateness and timeliness.

OBJECTIVE: To study the appropriateness of, and time taken, to transfer pediatric trauma patients in New South Wales to The Children's Hospital at Westmead (CHW), a pediatric trauma center. METHODS: All trauma patients transferred to CHW from June 2003 to July 2004 were included in the study. Indications and time periods relevant to the transfer of the patient from the referring institute were retrieved and analyzed. Pediatric and adult retrieval services were compared. RESULTS: Three hundred ninety-eight patients were transferred to CHW, of whom 332 were from the metropolitan region. Falls and burns were the commonest mechanism of injury. Burn was the commonest indication for transfer (107 of 398). Mean Injury Severity Score was eight. Nearly half the patients had minor injuries (Injury Severity Score<9). Patients spent an average of 5 hours at the referring hospital. Pediatric retrieval ambulances had significantly longer mean transfer times than did nonpediatric ambulance services with a total time spent of about 2.64 hours versus 1.30 hours, respectively. For aeromedical transfers, on the other hand, the difference between pediatric retrieval services and nonpediatric air ambulances was not significant. CONCLUSIONS: The majority of the patients transferred had minor injuries. Pediatric trauma patients spend considerable time in their referring hospitals. Pediatric retrieval services appear to take significantly longer to transfer patients than nonpediatric ambulance transfers even after allowing for patient age and injury severity. Although this did not result in mortality or morbidity, there appears to be considerable scope for a reduction in transfer times through better coordination of these services.

Ingested magnets and gastrointestinal complications.

Multiple magnet ingestion is an unexpected health hazard in children that can lead to significant gastrointestinal morbidity. The magnets are attracted to each other across the bowel wall and this may lead to pressure necrosis, resulting in perforation, fistula formation, and/or intestinal obstruction. We report herein a case of small bowel obstruction following ingestion of two magnets. The public and clinicians should be aware of the health hazard of such devices.